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Otodental dysplasia


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									“Otodental”                           dysplasia
          Ren-Jye Chen, B.D.S.,* Horng-Sen Chen, B.D.S.,** Li-Min Lin, D.D.S., M.S., Ph.D.,***
          Cheng-Chung Lin, B.D.S.,****  and Ronald J. Jorgenson, D.D.S., Ph.D.,*****
          Kaohsiung, Taiwan, Republic of China, and San Antonio, Texas


          The case of a 3’%year-old    Chinese boy with the dental abnormalities  of “otodental”  dysplasia is
          reported. Hearing was normal. Dental anomalies consisted of delayed eruption of globe-shaped      molars,
          bulbous deciduous canines, and double pulp chambers in the molars. Radiographs taken 4 years later
          showed taurodontic molars, supernumerary    microdontic teeth, retarded formation of premolars, and
          probable aplasia of the mandibular second premolars.
          (ORAL SURC ORAL MED ORAL PATHOL 1988;66:353-8)

I   n 1972 Levin and Jorgenson’ reported a kindred of
 !tali~n ancestry with mu!tiple dental anomalies and
 associated sensorineural hearing loss. The disorder
 segregated as an autosomal dominant. The authors
 first coined the term familial       otodentodysplasia,’
 then otodental dysplasia21 3 for this condition. The
 dental manifestations of the syndrome consisted of
 large, globe-shaped molars, absent or small premo-
 lars, and delayed eruption of affected teeth. Howev-
 er, deciduous and permanent incisors were normal in
 shape and size. High-frequency hearing loss was
 detected by pure- tone air-conduction audiometry in
 individuals with the dental abnormalities. The age at
 onset of hearing loss varied from early childhood to               Fig.   1. Dental cast of mandibular dentition of patient at
middle age.                                                         age 3?& years. Deciduous canine was bulbous, with groove
                                                                    on lingual surface. Deciduous first molar was globe
    Subsequently Gundlach and coauthors4-6reported
                                                                    shaped, with a pit in central region of occlusal surface;
another affected family. They proposed the terms                    many developmental grooves radiated from this pit and
globodontia for the globe-shaped dental deformity                   divided crown into lobules of different sizes. Erupting
and otodental syndrome for the concurrence of the                   deciduous second molar had just perforated the overlying
dental and hearing anomalies.                                       gingiva (arrows).
    Review of the literature revealed reports of oto-
dental dysplasia in five kindreds, each with several
affected individuals, and in two sporadic caseV; all
were in Westerners. We report the case of a Chinese                 CASE REPORT
boy with otodental dysplasia who had normal hear-                      This 3’/12-year-old Chinese boy was first seen at the
ing. It is the first reported case of this syndrome in an           Dental Clinic of Kaohsiung Medical College because of
Oriental.                                                           delayed eruption of his posterior teeth. The boy was the
                                                                    product of an uncomplicated, full-term pregnancy for a
                                                                    30-year-old couple. Birth weight was 3.1 kg. During
*Lecturer, Department of Oral Pathology; presently studying in
Department of Orthodontics, Loyola University of Chicago.           pregnancy his mother had no illnesses and took no medica-
**Lecturer, Department of Pedodontics.                              tions. Except for the dental problem, the boy’s develop-
***Professor and Chairman, Department of Oral Pathology.            ment was normal.
****Associate   Professor, Department of Oral Pathology.               His first tooth, a maxillary central incisor, erupted at
*****Professor,  Department of Pediatric Dentistry.                 age 4 months. At the time of oral examination           the

354    Chen et al.                                                                                             Oral Surg
                                                                                                          September 1988

        Fig. 2. Deciduous incisors are normal in shape. Each deciduous canine shows a spotlike defect on labial
        surface (arrows).

        Fig. 3. Panoramic radiograph of jaws at age 3‘l/l2 years shows enormous crowns and apparent duplicated
        pulp chambers of deciduous molars.

deciduous mandibular right first molars were not yet           1). Radiographs of the patient’s jaws showed that all
erupted. The deciduous mandibular second molars were           developing deciduous molars had enormous crowns (Fig
just erupting and penetrating the overlying gingiva (Fig.      3). Each of the crowns of the deciduous mandibular second
 1). Alignment of erupted teeth was normal. Structure of       molars appeared to be either duplicated or fused with a
the incisors was normal (Fig. 2). However, the canines and     supernumerary component into a twin crown. The primor-
molars were markedly deformed and large (Fig. 1).              dia of the mandibular premolars, the maxillary second
Canines were bulbous, and each had a vertical groove           premolars, and the permanent second molars were undis-
extending from the cusp tip onto the lingual surface. Each     cernible.
canine had an enamel defect, 1.5 X 1.5 mm in size, on the         The boy was reexamined at age 7% years. His decidu-
cervical third of the labical surface (Fig. 2).                ous molars were reported to have fully erupted by age 6
   Erupted molars were bulbous. A pit was located in the       years. His face was normal and symmetric (Fig. 4). His
center of the occlusal surface of each molar; many devel-      hair was straight and clinically appeared of normal densi-
opmental grooves radiated from these pits onto the buccal,     ty. The permanent maxillary central incisors and the
lingual, and proximal surfaces toward the cervical area,       permanent mandibular incisors had replaced their decidu-
and divided each crown into lobules of different sizes (Fig.   ous predecessors.Nevertheless, none of the permanent first
Volume 66                                                                               ‘Otodental”   dysplasia     355
Number 3

                                                             Fig. 5. Patient’s dentition reveals abnormally shaped
                                                             deciduous molars and two supernumerary microdontic
Fig. 4. Patient at age 7% years. His face was normal         teeth on palatal side of deciduous maxillary left molars.
and symmetric.

Table I. Comparison of the mean mesiodistal crown
diameters of deciduous teeth in Chinese boysI and
our patient
                                  Present case
        Chinese boys           Left side     Right side
           (mm)                 (mm)            (mm)
    Central incisor    6.61       7.12            7.30
    Lateral incisor    5.52       5.18            5.25
    Canine             6.57       7.92            8.02
    First molar        7.45       9.47            9.18
    Second molar       9.39      12.43           12.10
   Mandible                                                  Fig. 6. Heavy dental plaque was deposited on con-
    Central incisor     4.52      4.15            4.17       stricted cervical area of deciduous mandibular right first
    Lateral incisor     4.74      4.45            4.42       molar. Also note spotlike defects on labial surfaces of
    CAnine              5.94      8.35            8.40       canines of both jaws.
    First molar         8.08     14.25           14.38
    Second molar       10.26     15.48           15.47
                                                             showed incipient caries in the developmental grooves.
                                                             Heavy dental plaque was found on the constricted cervical
molars had erupted. Although all posterior teeth were        area of the mandibular first molars (Fig. 6). In addition,
large and deformed, alignment was good (Fig. 5). Attrition   conically shaped supernumerary microdontic teeth were
was noted on the occlusal surfaces of the molars. Amalgam    present on the palatal side of each deciduous maxillary left
restorations had been placed in the mandibular right first   molar (Fig. 5). Occlusion was compatible with an Angle
and left second molars. However, all mandibular molars       Class III molar relationship.
356    Chen et al.                                                                                             Oral Surg
                                                                                                          September 1988

        Fig. 7. Panoramic radiograph of jaws at age 7 years and 10 months shows both taurodontic configuration
        and vertical septum in pulp chamber of mandibular molars (arrows]. Primordia of mandibular second
        premolars are still absent.

    Dental radiographs showed the roots of the deciduous       tal dysplasia, because he did not have the typical
 molars to be disproportionately short compared to their       dental abnormalities of this syndrome.
crowns (Fig. 7). Developing permanent mandibular first            Dental anomalies and hearing loss usually co-exist
molars were taurodontic. A vertical septum was noted in        in individuals with otodental dysplasia. However,
the pulp chamber of each mandibular deciduous second           some patients may have the dental anomaly
and permanent first molar; thus the pulp chambers
appeared duplicated. The primordia of the mandibular
                                                                            because onset of hearing loss may vary
                                                               only, 3*5*‘,1’
second premolars and the permanent mandibular right            from early childhood to middle age.3,7Thus it is
second molar still could not be seen.                          likely that hearing loss will develop later in life in our
    Mesiodistal crown diameters of the deciduous teeth were    patient. The proband of the family reported by
measured with a vernier caliper (measuring to 0.05 mm)         Jorgenson and colleagues’ was first thought to have
on poured casts by the method of Moorrees.14Data were          normal hearing but proved to have progressive hear-
compared with the mean for unaffected Chinese boys.‘5          ing loss at 6’s~ years of age.l’ Another patient who
Mesiodistal crown diameters of all deciduous canines and       had the dental abnormalities of otodental dysplasia
molars of this boy were much greater than normal (Table        was depicted with a photograph of the teeth only;
I). IIowever, the boy’s deciduous maxillary lateral incisors   hearing status was not mentioned.‘O
and all deciduous mandibular incisors were smaller than           Hearing loss (hearing threshold greater than 25 B
those of unaffected Chinese boys.
                                                               in one or both ears) in affected individuals may be
   His parents and a S-year-old sister were examined; they
had no morphologic dental anomalies or congenitally            found at frequencies from 500 to 3000 Hz3*5,9 at     or
absent teeth. Other relatives reportedly had normal teeth      all tested frequencies. 12-13  Cook and coauthors”
and normal hearing but were not examined.                      reported that the sensorineural hearing loss may
   Pure-tone air-conduction audiometry was performed on        plateau by the fourth decade and result in a severe,
the patient, his parents, and his sister in a soundproof       flat, and bilaterally symmetric loss. They also pro-
booth. Results were normal. The father’s hearing thresh-       posed that the lesion was located in the cochlea but
old, although normal at the other frequencies, dropped to      that the mechanism for developing the loss was
65 dB at 8000 Hz. However, he had worked in a noisy            unknown. Hearing loss in our patient’s father may
factory for many years.                                        have been caused by the noisy environment he had
                                                               worked in for many years. He had no hearing-
                                                               impaired relatives.
   Table II summarizes previous reports of otodental              Other dental anomalies have been noted in persons
dysplasia as well as our own. Autosomal domi-                  with otodental dysplasia. Eruption of affected decid-
nant inheritance was demonstrated in all reported              uous canines and molars is often delayed until after
kindreds in which several individuals were af-                                       and
                                                               age 2 years,2v3s5-7 complete eruption of the
fected.‘-7*9. Griffin’sI patient did not have otoden-          deciduous dentition may be delayed, as in the boy
Volume 66                                                                                              “Otodental”       dysplasia     357
Number 3

Table II. Kindreds or sporadic cases of otodental dysplasia reported by other investigators and by present
                                                                                                        No. of proved
                                                           Ancestry of                                  affected cases
        Family                                               affected                Mode of
        number                Investigator                  patient(s)             inheritance        HL and DA              DA only

         1                Levine et al.‘-’                 Italian                     AD                 26                    3
         II               Gundlach et aL4”                 Polish                      AD                  2                    1
         III              Jorgenson et al.’                German                      AD                 13                    17
         IV               Rapp and                         British                     AD                  5
         V                Magnusson and                    ?                            ?                 ?                     I
         VI               Stewart et al.”                  Irish                        ?                                       1
         VII              Beck-Mannagetta                  Austrian                    AD                  3
                             et al.‘*. I3
         VIII             Chen et al.*                     Chinese                      ?                                       1

HL = coincide of hearing loss; DA = dental anomaly; AD = autosomal dominant.
‘Present authors.
tThe proband was proved to have hearing loss subsequent to the original report.”

 reported here, until age 6 years.9,‘2Eruption of the                          premolars should be discernible on radiographs by
 permanent posterior teeth may also be delayed.2~9~‘2                          ages 2 and 3 years, respectively,‘* and a tentative
 Winter9 reported a woman with the syndrome in                                 diagnosis of aplasia of a premolar can be made by
 whom the mandibular permanent first molar did not                             age 6 years.” In our patient calcification of the
 erupt until age 29 years.                                                     crowns of the maxillary premolars and the mandibu-
    Canines and molars have been reported to be                                lar first premolars was delayed until after 4 years of
 deformed and large in this syndrome.2x3. We   5-‘3                            age. His mandibular second premolars still cannot be
 confirmed this finding by measuring the mesiodistal                           seen on radiographs at age 7% years and may be
 diameters of these teeth on our patient’s dental casts                        congenitally absent.
 (Table I). Incisors have been reported normal in                                 Radiographic features of the teeth in otodental
 shape and size but have never been previously                                 dysplasia are striking. Findings consist of large and
 measured. In our patient mesiodistal crown diame-                             apparently duplicate pulp chambers, pulp calcifica-
 ters of the deciduous maxillary lateral incisors and                          tions, and taurodontic molars.2.3’5Beck-Mannagetta
 the deciduous mandibular incisors were found to be                            and associates’2disclosed by ground section that the
 smaller than those of Chinese boysI without the                               apparently duplicated pulp chambers each had a
syndrome.                                                                      longitudinal dentin septum. Witkop and coauthors5
    Many previous reports of otodental dysplasia,5-‘0                          reported that the taurodontic molars were due to
as well as our study, describe an enamel defect on the                         enlarged crowns and pulp chambers coronal to unaf-
labial surface of the canines. Witkop and coauthors5                           fected roots rather than apically placed pulp floors,
disclosed, by ground section, that the defect con-                             as is seen in true taurodontism. The structure of our
sisted of reduced enamel thickness, enamel hypoma-                             patient’s deciduous molars is compatible with this
turation, and irregularities of adjacent dentinal                              hypothesis, but the configuration of his developing
tubules. There may also be considerable variation in                           permanent mandibular first molars fulfills the crite-
the shape of affected molars among patients. Some                              ria of true taurodontism.20
patients have molars that are dome shaped with                                    Other anomalies that may be associated with
absent or shallow fissures and cusps8-“; other                                 otodental dysplasia are congenital coloboma of the
patients, like ours, may have molars with multiple                             em9 complex odontoma,‘2xI3 and supernumerary
                       5-7, I3
prominent lobules.2*3, 12* The reason for these                                microdontic teeth.9 Our patient had supernumerary
differences is unknown. In addition, deformities of                            microdontic teeth.
the deciduous posterior teeth are more severe than                                Histologic examination of abnormal teeth in oto-
those of the permanent dentition.                                              dental dysplasia has revealed aberrant morphodiffer-
   Small or absent premolars may be the only dental                            entiation but normal histodifferentiation.5.12 Levin
abnormality in otodental dysplasia.3 In the normal                             and coworkers3 have suggested that a defect in the
individual dental crypts of the first and second                               dental papilla could account for the multiple con-
                                                                                                                             Oral Surg
                                                                                                                       September 1988

                                                                      : 1. Stewart DJ, Kinirons MJ. Globodonria--a       rarely reported
                                                                           dental anomaly. Br Dent J 1982;152:287-8.
                                                                      12. Beck-Mannagetta     J. Miiller H. Richter E. Donath K. Odon-
                                                                           tome und pantonale Horstiirung bei otodentalem Syndrom.
                                                                           Dtsch Zahnarztl Z 1984;39:232-41.
                                                                      13. Beck-Mannagetta     J, Miiller H, Richter E, Donath K. Eine
 1. Levin LS. Jorgcna~)n RJ. f’amiliai otodentodq~pla~ia: ‘t “new.‘
                                                                           Familie mit otodentaler Dysplasie, Odontomen und pantonal-
    syndrome. [Cited in program abstract of the American
                                                                           er Horstiirung.   Dtsch Z Mund Kiefer Gesichts Chir
    Society of Human Genetics meeting. p, hla: October 1972
 , Levin LS. Jorgrnxnt R.I. Otodentai dysplasia: a prc\, iousl!       14. Moorrees CFA. The dentition of the growing child. London:
                                                                           William Clowes and Sons Limited, 1959:79.
    undescribed syndrome. Birth Defects 1974;10:3 1O-2.
                                                                      15. Tsai GS. The Chinese dentition: III. Mesiodistal diameters of
 3 Levin LS. Jorgenson RJ, Cook RA. Otodcntal dysplasia: a
                                                                           permanent and decidous teeth. Taiwan I Hsuch Hui Tsa Chih
    “new” ectodermal dysplasia. Clin Genet 1975;8: 136-44.
 3. Gundlach KKII, Witkop CJ Jr, Strced WJ. Sauk JJ Jr.               16. Griffin GJ. Case report: a possible variant of otodental
    Globodontia: a new inherited anomaly of tooth form [Ab-                syndrome. J Paediatr Dent 1985;1:27-33.
    stract]. Presented at American Academy of Oral Pathology          17. Cook RA, Cox JR, Jorgenson RJ. Otodental dysplasia: a five
    Annual Meeting, 1974. New8 Orleans.                                    year study. Ear Hear 1981;2:90-4.
 3 Witkop CJ Jr. Gundlach KKH, Streed WJ, Sauk JJ Jr.                 18. Jorgenson RJ. Clinician’s view of hypodontia. J Am Dent
    Globodontia in the otodental syndrome. ORAL SURG ORAL                  Assoc 1980;101:283-6.
    MED OR,\L PATHOL 1976;4 1:472-83.
                                                                      19. Cunat JJ, Collord J. Late developing premolars: a report of
 6. Gundlach KKH, Witkop CJ .Jr. Globondontie-           eine neuc         two cases. J Am Dent Assoc 1973;87:183-5.
    erbliche Zahnformanomalie.        Dtsch Zahnlrztl    2, 1977:     20. Mena CA. Taurodontism.         ORAL SURG ORAL MED ORAL
    32: 194-6.                                                            PATHOL 1971;32:812-22.
 7. Jorgenson RJ. Marsh SJ, Farrington FH. Otodental dyspla-
    sia. Birth Defects 1975:ll:I 15-9.
 8. Rapp R. Winter GB. A colour atlas of clinical conditions in       Reprint requests to:
    paedodontics. London: Wolfe Publications Ltd, 1979:39.            Dr. Ren-Jye Chen
 9. Winter GB. The association of ocular defects with the             P.O. Box 795
    otodental syndrome. J Int Assoc Dent Child 1983;14:83-7.          Department of Orthodontics
10. Magnusson BO, Persliden B. Development and its aberra-            School of Dentistry
    tions. In: Magnusson BO, ed. Pedodontics: a systematic            Loyola University of Chicago
    approach. 1st ed. Copenhagen: PJ Schmidt Vojens Co,               2160 S. 1st Ave.
    1981:112-3.                                                       Maywood, IL 60153

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